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Dergiler / Erciyes Medical Journal / 2022 / Cilt: 44 - Sayı: 2

Progressive Familial Intrahepatic Cholestasis in a Newborn Treated with Liver Transplantation

Hakan ONGUN
İpek ACARBULUT
Celal SAĞLAM
Nihal OYGÜR
Kıymet ÇELİK
Sayfa
232–234
Yayın Tarihi
2022-10-11
DOI
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Conclusion: As in this PFIC2 case, PFIC can occasionally be very severe and rapidly progress to liver failure and require liver transplantation in the neonatal period.

📌 3 Maddede Bu Makale

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Amaç & Hipotez:Background: Progressive familial intrahepatic cholestasis (PFIC) is a severe cholestatic liver disease often requiring a liver transplant (LT).
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Temel Bulgular & Veri:After excluding other common causes of neonatal cholestatic jaundice in the differential diagnosis and considering the combination of the clinical and laboratory findings of hepatosplenomegaly and biopsy results, the patient was diagnosed with PFIC type 2 (PFIC2).
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Sonuç & Katkı:Conclusion: As in this PFIC2 case, PFIC can occasionally be very severe and rapidly progress to liver failure and require liver transplantation in the neonatal period.
🔬Tür:Araştırma Makalesi
📍Kapsam:Genişletilmiş Akademik Örneklem
⚙️Değişkenler:Alan Parametreleri

Abstract

Background: Progressive familial intrahepatic cholestasis (PFIC) is a severe cholestatic liver disease often requiring a liver transplant (LT). This report describes PFIC in a newborn with jaundice and hepatosplenomegaly treated with LT in the neonatal period. Case Report: A female infant was referred to the neonatal intensive care unit on the first day of life due to direct hyperbilirubinemia and thrombocytopenia. After excluding other common causes of neonatal cholestatic jaundice in the differential diagnosis and considering the combination of the clinical and laboratory findings of hepatosplenomegaly and biopsy results, the patient was diagnosed with PFIC type 2 (PFIC2). LT was performed on the 33rd day of life once the infant had achieved a weight of 3400 g. Conclusion: As in this PFIC2 case, PFIC can occasionally be very severe and rapidly progress to liver failure and require liver transplantation in the neonatal period.

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Bu Makaleye Atıf Yap

ONGUN, H., ACARBULUT, İ., SAĞLAM, C., OYGÜR, N., ÇELİK, K. (2022). Progressive Familial Intrahepatic Cholestasis in a Newborn Treated with Liver Transplantation. Erciyes Medical Journal, Cilt: 44 - Sayı: 2, 232–234. https://ajindex.com/articles/502150

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Kaynakça (7)

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